Key result
Familial atrial myxoma resection in Carney complex shows ~67% recurrence requiring long-term surveillance.
Case Report (n=3)
No
Familial atrial myxoma carries a high risk of recurrence, highlighting the need for interval echocardiographic surveillance and family screening.
Supports surveillance in familial myxoma; leaves open optimal protocols pending larger cohorts.
Carney complex accounts for up to two-thirds of familial cardiac myxoma. It is a rare autosomal dominant syndrome, which is also characterized by multiple mucocutaneous lesions and endocrine tumors. We report on three first-degree relatives who underwent surgical resection at the same Australian tertiary institution. One patient re-presented with a recurrent tumor at an interval of 6 years. In this context, the role of interval surveillance, family screening, and genetic testing is explored. We recommend interval echocardiographic surveillance for affected individuals and first-degree relatives given the high risk of recurrence and the morbidity and mortality associated with cardiac tumors in any location.
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Schmidt et al. (2017) conducted a case report in Familial Atrial Myxoma / Carney complex (n=3). Surgical resection was evaluated on Tumor recurrence and surgical outcomes. Three first-degree relatives with familial atrial myxoma associated with Carney complex underwent successful surgical resection, with two experiencing recurrences, highlighting the need for long-term surveillance.
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